Rett syndrome: analysis of deaths in the British survey.
The mortality rate in Rett syndrome is 1.2% per annum, 48% of deaths occurring in debilitated people, 13% from natural causes, 13% with prior severe seizures and 26% sudden and unexpected. Respiratory dysrhythmias were usually present. Neuropathology confirmed reductions in cortical dendrites and in one case immaturity of cardiac conducting tissues.
Kerr
A M
AM
Department of Psychological Medicine, Gartnavel Royal Hospital, Glasgow, Scotland.
Armstrong
D D
DD
Prescott
R J
RJ
Doyle
D
D
Kearney
D L
DL
D002423:Cause of Death; D005260:Female; D006801:Humans; D011159:Population Surveillance; D011446:Prospective Studies; D012120:Respiration Disorders / Q000401:mortality; D015518:Rett Syndrome / Q000401:mortality* / Q000473:pathology; D012720:Severity of Illness Index; D006113:United Kingdom / Q000453:epidemiology